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Tongue Strength and Speech in Oculopharyngeal Muscular Dystrophy
Amy T. Neel, Phyllis M. Palmer., Gwyneth Sprouls, and Leslie A. Morrison

Oculopharyngeal muscular dystrophy (OPMD) is a rare genetic myopathy occurring in Hispanic northern New Mexicans (Becher et al., 2001). OPMD is characterized by progressive ptosis and dysphagia that develops in the fifth decade of life and may also be accompanied by flaccid dysarthria. Fatty replacement of muscle tissue has been observed in some speech muscles. The purpose of this study is to document speech problems associated with OPMD and to relate them to deficits in tongue strength and endurance. The three individuals with OPMD who have been studied so far demonstrated markedly reduced tongue strength and endurance while speech intelligibility was relatively preserved. There was evidence of reduced diadochokinetic rates and slower articulation rates in sentences compared to controls suggesting that these individuals may be slowing their speech in order to maintain articulatory accuracy. Continued study of speech characteristics in OPMD is underway including detailed acoustic analysis to determine how speech is affected by tongue weakness in this population.

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